title: Progressive multi-organ expression of immunoglobulin G4-related disease: A case report.
abstract: A 63-year-old Caucasian man presented with a cholestatic syndrome, renal failure and arthralgias. A laboratory examination revealed high immunoglobulin G (IgG) and IgG4 levels (5.95 g/L; normal range: 0.08-1.4 g/L), pointing to a diagnosis of systemic IgG4-related disease, with definite radiological evidence of biliary and pancreatic expression, and plausible renal, articular, salivary and lacrimal glands involvement. Due to the rarity of the condition, there are currently no random control trials to point to the optimal therapeutic approach. The patient has been on steroid therapy with the subsequent introduction of azathioprine, with a complete resolution of all symptoms, a rapid reduction to normalization of all blood tests, and a complete regression of the radiological picture. Our experience underlines the complexity of IgG4-related disease and its variable and sometimes progressive presentation, while pointing out the need for a careful and complete assessment for possible multi-organ involvement.
authors.affiliation: Marcello Maida, Fabio Salvatore Macaluso, Giuseppe Cabibbo, Nicola Alessi, Section of Gastroenterology, DIBIMIS, University of Palermo, 90127 Palermo, Italy.
authors.forename: Marcello
authors.initials: M
authors.lastname: Maida
authors.forename: Fabio Salvatore
authors.initials: FS
authors.lastname: Macaluso
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cabibbo
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lo Re
authors.forename: Nicola
authors.initials: N
authors.lastname: Alessi
keywords: Autoimmune cholangitis; Autoimmune pancreatitis; Immunoglobulin G4 related-disease; Renal failure